Long-Term Follow-Up of Significant Improvement After CAPTEM Treatment for Rare Adrenocorticotropin-Producing Cardiac Neuroendocrine Tumor

Frontiers in Endocrinology

Renzhi Wang¹  Hao Wang²  Xin Cheng²  Chunmei Bai³  Yuejuan Cheng³  Yuan Li⁴  Zhiyong Liang⁴  Boju Pan⁴  Yu Xiao⁴  Jian Sun⁴  Yining Wang⁵  Tao Yuan⁶  Huijuan Zhu⁶  Xiaoping Xing⁶  Zhaolin Lu⁶  Huabing Zhang⁶  Lin Lu⁶  Naishi Li⁶  Qingqing Meng⁷ 

[1] Department of Neurosurgery, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China;Department of Nuclear Medicine, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China;Department of Oncology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China;Department of Pathology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China;Department of Radiology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China;Key Laboratory of National Health Commission, Department of Endocrinology, Peking Union Medical College, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China;School of Medicine, Tsinghua University, Beijing, China;

关键词: Cushing syndrome;    ACTH syndrome;    ectopic;    cardiac neoplasms;    neuroendocrine tumor;    chemotherapy;   

DOI  :  10.3389/fendo.2019.00713

来源: DOAJ

【 摘 要 】

Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a rare cause of Cushing syndrome. If routine imaging examinations cannot identify the source of ACTH production, long-term follow-up observation is necessary to determine the etiology. We present the case of a middle-aged male with gradual weight gain and a Cushingoid appearance over 4 years; he provided written informed consent. Laboratory and endocrine tests strongly suggested EAS, although the origin was not detected by multiple imaging methods. Bilateral adrenalectomy was performed to prevent severe complications in the patient. Two and a half years later, a cardiac mass 18 × 23 × 27 mm in size at the junction between the anterior wall of the left ventricle and the middle septum was found together with multiple bone metastases by 18F-FDG PET/CT, while the 68Ga-DOTATE PET/CT findings were negative. Biopsy of the lumbar vertebrae revealed a neuroendocrine tumor (NET) with positive ACTH staining. The patient underwent chemotherapy by CAPTEM, resulting in shrinkage of the cardiac mass and a significant decrease in the ACTH level. In the case of EAS with an unusual cause, long-term follow-up observation is necessary to determine the source of ACTH production. Cardiac NETs are quite rare in EAS, so treatment selection was also challenging. CAPTEM chemotherapy proved effective in controlling the progression of tumor growth and decreasing the ACTH level in this patient.

【 授权许可】

Unknown